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Lyell Syndrome

definition

Toxic epidermal necrolysis is an acute severe bullous cutaneous disease characterized by extensive areas of skin necrosis accompanied by a systemic toxic condition. It may occur in all age groups. In most children TEN develops in the form of blistered erythematous patches and plaques which evolve within hours to extensive areas of skin necrosis with severe epidermolysis. In few children the lesions will begin with denuding of oral mucosa followed by extensive areas of skin necrosis but with some areas of normal skin in-between. Malaise and fever accompany marked skin tenderness and erythema. The rash rapidly becomes confluent over large areas and forms large slack blisters. Mucous membranes may be extensively involved. Because of the extensive area of eroded skin, large amounts of body fluid are lost with consequent distrubances of the electrolyte and fluid balance. A number of drugs have been incriminated as causative agents including pyrazolones, hydantoin derivatives, sulfonamides and barbiturates.